{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T17:11:22Z","timestamp":1793380282351,"version":"4.5.0"},"reference-count":30,"publisher":"BMJ","issue":"2","license":[{"start":{"date-parts":[[2021,9,28]],"date-time":"2021-09-28T00:00:00Z","timestamp":1632787200000},"content-version":"unspecified","delay-in-days":27,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100016220","name":"CTSA","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100016220","id-type":"DOI","asserted-by":"crossref"}]},{"name":"NIH\/NCATS","award":["UL1 TR002535"],"award-info":[{"award-number":["UL1 TR002535"]}]},{"name":"NIH","award":["K25 HL133481"],"award-info":[{"award-number":["K25 HL133481"]}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["Open Heart"],"accepted":{"date-parts":[[2021,8,27]]},"published-print":{"date-parts":[[2021,9]]},"abstract":"\n Aims<\/jats:title>\n \n Ventricular\u2013vascular coupling, the ratio between the right ventricle\u2019s contractile state (E\n es<\/jats:sub>\n ) and its afterload (E\n a<\/jats:sub>\n ), may be a useful metric in the management of paediatric pulmonary arterial hypertension (PAH). In this study we assess the prognostic capacity of the ventricular\u2013vascular coupling ratio (E\n es<\/jats:sub>\n \/E\n a<\/jats:sub>\n ) derived using right ventricular (RV) pressure alone in children with PAH.\n <\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n \n One hundred and thirty paediatric patients who were diagnosed with PAH via right heart catheterisation were retrospectively reviewed over a 10-year period. Maximum RV isovolumic pressure and end-systolic pressure were estimated using two single-beat methods from Takeuchi\n et al<\/jats:italic>\n (E\n es<\/jats:sub>\n \/E\n a<\/jats:sub>\n _(Takeuchi)) and from Kind\n et al<\/jats:italic>\n (E\n es<\/jats:sub>\n \/E\n a<\/jats:sub>\n _(Kind)) and used with an estimate of end-systolic pressure to compute ventricular\u2013vascular coupling from pressure alone. Patients were identified as either idiopathic\/hereditary PAH or associated PAH (IPAH\/HPAH and APAH, respectively). Haemodynamic data, clinical functional class and clinical worsening outcomes\u2014separated into soft (mild) and hard (severe) event categories\u2014were assessed. Adverse soft events included functional class worsening, syncopal event, hospitalisation due to a proportional hazard-related event and haemoptysis. Hard events included death, transplantation, initiation of prostanoid therapy and hospitalisation for atrial septostomy and Pott\u2019s shunt. Cox proportional hazard modelling was used to assess whether E\n es<\/jats:sub>\n \/E\n a<\/jats:sub>\n was predictive of time-to-event.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n In patients with IPAH\/HPAH, E\n es<\/jats:sub>\n \/E\n a<\/jats:sub>\n _(Kind) and E\n es<\/jats:sub>\n \/E\n a<\/jats:sub>\n _(Takeuchi) were both independently associated with time to hard event (p=0.003\u2009and p=0.001, respectively) and when adjusted for indexed pulmonary vascular resistance (p=0.032\u2009and p=0.013, respectively). Neither E\n es<\/jats:sub>\n \/E\n a<\/jats:sub>\n _(Kind) nor E\n es<\/jats:sub>\n \/E\n a<\/jats:sub>\n _(Takeuchi) were associated with time to soft event. In patients with APAH, neither E\n es<\/jats:sub>\n \/E\n a<\/jats:sub>\n _(Kind) nor E\n es<\/jats:sub>\n \/E\n a<\/jats:sub>\n _(Takeuchi) were associated with time to hard event or soft event.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n \n E\n es<\/jats:sub>\n \/E\n a<\/jats:sub>\n derived from pressure alone is a strong independent predictor of adverse outcome and could be a potential powerful prognostic tool for paediatric PAH.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1136\/openhrt-2021-001611","type":"journal-article","created":{"date-parts":[[2021,9,28]],"date-time":"2021-09-28T15:00:15Z","timestamp":1632841215000},"page":"e001611","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":15,"title":["Ventricular\u2013vascular coupling is predictive of adverse clinical outcome in paediatric pulmonary arterial hypertension"],"prefix":"10.1136","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2920-3464","authenticated-orcid":false,"given":"Melanie J","family":"Dufva","sequence":"first","affiliation":[{"name":"Bioengineering, University of Colorado Denver - Anschutz Medical Campus, Aurora, Colorado, USA"},{"name":"Cardiology, Children's Hospital Colorado, Aurora, Colorado, USA"},{"name":"Pediatrics, University of Colorado Denver School of Medicine, Aurora, Colorado, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dunbar","family":"Ivy","sequence":"additional","affiliation":[{"name":"Cardiology, Children's Hospital Colorado, Aurora, Colorado, USA"},{"name":"Pediatrics, University of Colorado Denver School of Medicine, Aurora, Colorado, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristen","family":"Campbell","sequence":"additional","affiliation":[{"name":"Cardiology, Children's Hospital Colorado, Aurora, Colorado, USA"},{"name":"Pediatrics, University of Colorado Denver School of Medicine, Aurora, Colorado, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aimee","family":"Lam","sequence":"additional","affiliation":[{"name":"Bioengineering, University of Colorado Denver - Anschutz Medical Campus, Aurora, Colorado, USA"},{"name":"Cardiology, Children's Hospital Colorado, Aurora, Colorado, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam","family":"Rauff","sequence":"additional","affiliation":[{"name":"Bioengineering, University of Colorado Denver - Anschutz Medical Campus, Aurora, Colorado, USA"},{"name":"Department of Bioengineering, University of Utah Health, Salt Lake City, Utah, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karel T N","family":"Breeman","sequence":"additional","affiliation":[{"name":"Cardiology, Children's Hospital Colorado, Aurora, Colorado, USA"},{"name":"Paediatric Cardiology, University Medical Center Groningen Department of Cardiology, Groningen, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johannes M","family":"Douwes","sequence":"additional","affiliation":[{"name":"Paediatric Cardiology, University Medical Center Groningen Department of Cardiology, Groningen, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rolf M F","family":"Berger","sequence":"additional","affiliation":[{"name":"Paediatric Cardiology, University Medical Center Groningen Department of Cardiology, Groningen, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vitaly Oleg","family":"Kheyfets","sequence":"additional","affiliation":[{"name":"Bioengineering, University of Colorado Denver - Anschutz Medical Campus, Aurora, Colorado, USA"},{"name":"Cardiology, Children's Hospital Colorado, Aurora, Colorado, USA"},{"name":"Pediatrics, University of Colorado Denver School of Medicine, Aurora, Colorado, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kendall","family":"Hunter","sequence":"additional","affiliation":[{"name":"Bioengineering, University of Colorado Denver - Anschutz Medical Campus, Aurora, Colorado, USA"},{"name":"Cardiology, Children's Hospital Colorado, Aurora, Colorado, USA"},{"name":"Pediatrics, University of Colorado Denver School of Medicine, Aurora, Colorado, 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\u201cEstimation of right ventricular isovolumic pressure from a single ejecting beat in experimental pulmonary hypertension\u201d PhD Thesis Vrije Universiteit Amsterdam; 2012."},{"key":"2025081709415949000_8.2.e001611.15","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2018.12.076"},{"key":"2025081709415949000_8.2.e001611.16","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2016.05.015"},{"key":"2025081709415949000_8.2.e001611.17","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01916-2018"},{"key":"2025081709415949000_8.2.e001611.18","doi-asserted-by":"publisher","DOI":"10.1093\/cvr\/4.1.23"},{"key":"2025081709415949000_8.2.e001611.19","doi-asserted-by":"crossref","first-page":"ii23","DOI":"10.1136\/heartjnl-2014-307340","article-title":"Hemodynamic assessment and acute pulmonary vasoreactivity testing in the evaluation of children with pulmonary vascular disease. expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. the 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